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Liver Diseases
Published in George Feuer, Felix A. de la Iglesia, Molecular Biochemistry of Human Disease, 2020
George Feuer, Felix A. de la Iglesia
A similar hepatic excretory defect is found in the Rotor syndrome except that there are no pigment bodies in hepatocytes. The liver structure is apparently normal although the number of pericanalicular lysosomes is increased. Rotor syndrome is a familial disorder and is associated with elevation of conjugated serum bilirubin. There is a mild jaundice which may be worsened with physical stress, chronic emotional problems, and infections. Abdominal pain is absent.
Hepatobiliary Surgery
Published in Gozie Offiah, Arnold Hill, RCSI Handbook of Clinical Surgery for Finals, 2019
➢ HepaticHepatic unconjugated hyperbilirubinaemia.■ Gilbert’s■ Crigler-Najjar syndromes.Hepatic conjugated hyperbilirubinaemia.■ Viral hepatitis.■ Alcoholic liver disease.■ Toxic drug jaundice.■ Metastatic disease.■ Dubin-Johnson Syndrome■ Rotor Syndrome
A Review on Ethnobotany of Hepatoprotective Plants of India
Published in T. Pullaiah, K. V. Krishnamurthy, Bir Bahadur, Ethnobotany of India, 2017
Hereditary jaundice or pure cholestasis: Gilbert’s syndrome, Dubin- Johnson syndrome, Crigler-Najjar syndrome, Rotor syndrome are some of the hereditary jaundice types usually observed. Gilbert’s syndrome and Crigler-Najjar syndrome are examples of hereditary non-hemolytic unconjugated hyper bilirubinemia, whereas Dubin-Johnson syndrome and Rotor’s syndrome are conditions with hereditary conjugated hyperbilirubinemia.
Inhibitory Effects of Bilirubin on Colonization and Migration of A431 and SK-MEL-3 Skin Cancer Cells Compared with Human Dermal Fibroblasts (HDF)
Published in Cancer Investigation, 2021
Javad Saffari-Chaleshtori, Ali Shojaeian, Esfandiar Heidarian, Sayed Mohammad Shafiee
Bilirubin is a tetrapyrrole and an endogenous catabolic product that is formed during heme catabolism. After lysis of the erythrocytes in the spleen and liver, macrophages remove the hemoglobin and separate the heme group. Subsequently, heme oxygenase converts heme to biliverdin that in turn is converted to unconjugated bilirubin by biliverdin reductase. Unconjugated bilirubin combines with glucuronic acid to form conjugated bilirubin in the liver and is then excreted in bile (11). The normal level of (unconjugated) bilirubin under physiological conditions is around 0.2 mg/dl (3.42 µM) (12). Bilirubin is a lipophilic compound and tends to cross the blood-brain barrier (13). This compound, at high concentrations, can exert neurotoxic effects and therefore induce severe complications in neurons (14). Neonatal jaundice is the most common cause of bilirubinemia. The genetic causes of this condition include Gilbert-Meulengracht, Crigler-Najjar, and Rotor syndrome (12). Serum bilirubin concentrations lower than 12–14 mg/dl are tolerable while higher levels lead to mental retardation especially in neonates (15). However, the mutation in the UDP-glycuronyltransferase gene causes a natural increase in bilirubin up to 1–3 mg/dl (17–51 µM) in Gilbert-Meulengracht syndrome (16).
Genetic polymorphisms of human hepatic OATPs: functional consequences and effect on drug pharmacokinetics
Published in Xenobiotica, 2020
Yingmin Nie, Jingjie Yang, Shuai Liu, Ruiqi Sun, Huihui Chen, Nan Long, Rui Jiang, Chunshan Gui
As shown in Table 1, OATPs transport many important endogenous substances. Therefore, the malfunction of human hepatic OATPs could lead to some diseases. For instance, mutations in OATP1B1 and OATP1B3 genes could cause Rotor syndrome due to the loss of function of OATP1B1 and 1B3 to transport bilirubin (van de Steeg et al., 2012). It was reported that OATPs have different expression levels in normal tissues and in cancer (Obaidat et al., 2012) and thus could be potential biomarkers for some cancers such as pancreatic, colorectal and hormone-related cancers (Hays et al., 2013; Morio et al., 2018; Pressler et al., 2011). OATPs could also be associated with the outcome of cancer treatment and prognosis (Drenberg et al., 2016). In addition, many drugs are the substrates of hepatic OATPs. Therefore, OATPs are important targets for mediating the occurrence of drug–drug interactions. Changes of OATP’s function by other drugs or by genetic polymorphisms could result in the alteration of pharmacokinetic properties of their substrate drugs and in turn could lead to adverse drug responses.
Diagnostic criteria and contributors to Gilbert’s syndrome
Published in Critical Reviews in Clinical Laboratory Sciences, 2018
Karl-Heinz Wagner, Ryan G. Shiels, Claudia Anna Lang, Nazlisadat Seyed Khoei, Andrew C. Bulmer
Interestingly, Rotor syndrome is associated with elevated TB and DBIL; it is a consequence of reduced DBIL transport into hepatocytes, which is caused by reduced expression of organic anion transporters OATP1B1 or OATP1B3 in the basolateral membrane of hepatocytes [78]. An increased DBIL suggests either Rotor or Dubin-Johnson syndrome. Rotor syndrome can be differentiated from Dubin-Johnson syndrome by observing delayed plasma clearance of bromsulphthalein, increased urinary excretion of coproporphyrins, and a lack of granular hepatocyte pigmentation [67].